Centene Kanuma Coverage Update Prior Auth

Coverage Criteria

inv-01: Initial Therapy

Covered when ALL of the following are met

Initial Approval - LAL deficiency: 1. Diagnosis of LAL deficiency confirmed by enzyme assay demonstrating deficient LAL activity OR by presence of a LIPA gene mutation; 2. Age ≥ 1 month; 3. Documentation of member's current weight (in kg); 4. Requested dose meets one of the following: a) does not exceed 3 mg/kg every other week; OR b) for members with rapidly progressive disease presenting within the first 6 months of life: i) does not exceed 3 mg/kg per week; OR ii) does not exceed 5 mg/kg per week upon documentation of suboptimal clinical response to 3 mg/kg per week (suboptimal clinical response defined as poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly).

inv-02: Continuation Therapy

Covered when ALL of the following are met

Continuation: 1. Member meets one of the following: a) currently receiving medication via Centene benefit or previously met initial approval criteria; OR b) currently receiving medication and continuity of care regulations apply in the member's state/product; 2. Member is responding positively to therapy as evidenced by clinical response which may include: a) for rapidly progressive disease presenting within first 6 months of life: continued survival; b) for all other members: decrease in LDL-c, non-HDL-c, or triglycerides; increase in HDL-c; normalization of ALT or AST; reduction in hepatic fat content, steatosis, or liver volume; 3. Documentation of member's current weight (in kg); 4. If request is for a dose increase, new dose does not exceed: a) 3 mg/kg every other week; OR b) for rapidly progressive disease presenting within first 6 months of life: i) 3 mg/kg per week; OR ii) 5 mg/kg per week upon documentation of suboptimal clinical response to 3 mg/kg per week (suboptimal clinical response defined as poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly).

Requests for use of sebelipase alfa (Kanuma) for indications that are not FDA‑approved and not specifically addressed in this policy will generally be considered outside the scope of coverage. Such requests may be approved only when the provider submits sufficient documentation of efficacy and safety in accordance with the applicable off‑label use policy (see CP.CPA.09 for Commercial, HIM.PA.154 for ICHRA/marketplace, and CP.PMN.53 for Medicaid) or when the member’s evidence of coverage explicitly authorizes the requested indication.

Coverage under this clinical policy is subject to the terms, conditions, exclusions and limitations of the member’s benefit documents and to applicable federal and state laws and regulations. The policy is intended as a guide to medical necessity and does not guarantee payment. When there is a conflict between this policy and legal or regulatory requirements (including state Medicaid provisions), applicable law and the member’s coverage documents govern.

Use of sebelipase alfa for indications outside the FDA‑approved label may be considered only when supported by documentation consistent with the Health Plan’s off‑label use policies (CP.CPA.09 for Commercial, HIM.PA.154 for ICHRA/marketplace, CP.PMN.53 for Medicaid) or when the member’s coverage documents allow the off‑label use. Providers seeking authorization for off‑label use must supply clinical evidence of efficacy and safety as described in those off‑label policies; absent such documentation, requests for non‑FDA indications may be denied.

Initial Therapy Specifics

inv-20: Initial therapy specifics

Initial approval requires diagnostic confirmation and appropriate dosing documentation

Diagnostic confirmation: Confirm diagnosis of lysosomal acid lipase (LAL) deficiency by one of the following: enzyme assay demonstrating deficient LAL activity OR detection of a pathogenic LIPA gene mutation.

Age requirement: Member is age ≥ 1 month at time of request.

Weight documentation: Provider must document member's current weight in kilograms for dose calculation.

Dosing within policy limits: Requested dose must conform to policy dosing limits: typical dosing does not exceed 3 mg/kg every other week; for rapidly progressive disease presenting within first 6 months of life allowable dosing includes up to 3 mg/kg per week, and up to 5 mg/kg per week only after documentation of suboptimal clinical response to 3 mg/kg per week.

Continuation Therapy

inv-21: Continuation criteria

Continuation therapy requires documentation of benefit and weight for dose calculations

Continuation - current therapy or prior approval: Member is currently receiving the medication via the health plan benefit or has previously met initial approval criteria, or continuity of care regulations apply.

Demonstrated positive response: Documentation of clinical response is required: for rapidly progressive infants (presenting within first 6 months): continued survival; for all other members: improvement in lipid parameters (decrease in LDL-c, non-HDL-c, or triglycerides; increase in HDL-c), normalization of ALT/AST, reduction in hepatic fat content, steatosis, or liver volume. Clinical response may be demonstrated by laboratory results, imaging, or clinician documentation.

Weight documentation for continuation: Provider must document member's current weight (kg) at the time of continuation request for dose verification.

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required. Requests must include diagnostic confirmation (enzyme assay demonstrating deficient LAL activity or LIPA gene mutation), member age (must be ≥ 1 month for initial approval), current weight in kilograms, and a requested dosing regimen that falls within policy limits (≤ 3 mg/kg every other week for standard disease; for rapidly progressive disease presenting within first 6 months of life, regimens up to 3 mg/kg/week or up to 5 mg/kg/week only after documented suboptimal response to 3 mg/kg/week).

Diagnostic confirmation: enzyme assay or LIPA mutation
Age requirement: ≥ 1 month for initial approval
Current weight (kg) must be documented
Requested dose must not exceed policy-specified maximums

Note

Prior Authorization and Coverage Governance

Coverage determinations follow Health Plan medical necessity criteria and member-specific benefit documents. Approval is subject to the terms, conditions, exclusions, and limitations of the member's evidence of coverage or contract. For Medicaid members, applicable state Medicaid provisions take precedence over this clinical policy.

Coding

HCPCS CodesHCPCS

J2840

Injection, sebelipase alfa, 1 mg

Hepatic steatosis improvement

ContextUsed as a measure of therapeutic response to Kanuma; reported alongside normalization of ALT/AST and reduction in liver volume

Step Therapy and Dose Escalation

Step	Dose (rapidly progressive disease)	When to escalate	Maximum after escalation
Start	1 mg/kg once weekly	If suboptimal clinical response (poor growth, deteriorating biochemical markers, or persistent/worsening organomegaly)	Up to 5 mg/kg once weekly after sequential escalation
Escalation 1	Increase to 3 mg/kg once weekly	If suboptimal clinical response after 1 mg/kg weekly	Up to 5 mg/kg once weekly after further escalation
Escalation 2	Increase to 5 mg/kg once weekly	If continued suboptimal clinical response after 3 mg/kg weekly	Maximum dose = 5 mg/kg once weekly

Quantity Limits

Sebelipase alfa quantity limits

Dosing basisQuantity limits are weight-based (mg/kg per dosing interval)

Product strengthSingle-use vial: 20 mg/10 mL

Coding referenceSebelipase alfa dosing and quantity calculations reference HCPCS code J2840 (in policy coding section)

Site of Care

Note

Administer IV (infusion center); site of care not specifically restricted

Intravenous administration is required per the dosing instructions; the policy does not restrict the specific site of care within this section (infusion center cited as the setting).

Administration route: IV infusion per dosing regimen
Policy does not impose a specific site restriction; infusion center is listed as the setting

Definitions

Suboptimal clinical response

DefinitionSuboptimal clinical response = poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly

Biochemical examplesIncludes worsening ALT, AST and/or parameters of lipid metabolism (e.g., LDL-c, triglycerides)

Use caseDefinition is used to justify dose escalation (e.g., increase from 1 mg/kg to 3 mg/kg or to 5 mg/kg for rapidly progressive disease)

Clinical policy definition

PurposeClinical policy provides guidance to assist in making coverage decisions and administering benefits

Not a contractIt does not constitute a contract or guarantee regarding payment or results

Background

Lysosomal acid lipase (LAL) deficiency is an inherited enzyme deficiency leading to accumulation of cholesteryl esters and triglycerides with clinical consequences that commonly include hepatomegaly, elevated liver enzymes, hepatic steatosis, and dyslipidemia. Enzyme replacement with sebelipase alfa (Kanuma) is indicated to replace deficient LAL activity and can result in improvement in biochemical markers, reduction in hepatic fat and liver volume, and improvement in lipid parameters; in rapidly progressive infantile disease, treatment can support survival. This clinical policy was developed to guide medical‑necessity determinations for sebelipase alfa and to define documentation, dosing, and continuation criteria used by the Health Plan.

Revision History

2026-04-10annual review - materialLatest

Updated initial approval duration from 6 months to 12 months and added ICHRA line of business (2Q 2026).

2025-03-10annual review

References reviewed and updated (2Q 2025).

2024-01-09annual review

OpenPayer

Sebelipase Alfa (Kanuma) therapy for lysosomal acid lipase deficiency